Brain tissue damage caused by the accumulation of misfolded, insoluble proteins leads to various forms of dementia (neurodegeneration).
Neurodegeneration is the breakdown of nerve cells in the central nervous system due to the accumulation of misfolded proteins. This often results in dementia, such as Alzheimer’s disease, various forms of frontotemporal dementia, Lewy body diseases (e.g., Parkinson’s disease), and prion diseases. The accumulation of misfolded proteins is insoluble and harmful. Protein misfolding can be caused by mutations, environmental factors, or (rarely) by infectious proteins from external sources. Additionally, the normal breakdown and clearance of brain proteins may be impaired. This is explained by neuropathologist Annemieke Rozemuller in her farewell lecture.
The neuropathologist diagnoses brain tissue after death and works closely with clinicians, radiologists, and clinical chemists to provide crucial feedback, while also collaborating with the brain bank. This enables fundamental scientists, who have access to an increasing number of techniques, to study disease processes. In this way, we improve diagnostics, gain new insights for future therapies, and discover new diseases.